Von-Hippel Lindau disease

Case contributed by Vikas Shah


2 weeks of headache with blurred vision.

Patient Data

Age: 20 years
Gender: Male

CT head


5 cm cystic posterior fossa lesion with enhancing 1 cm nodule superiorly. Mild surrounding edema. Signs of early hydrocephalus. No other lesions. Features suggest hemangioblastoma.

The lesion was surgically excised and confirmed to be a hemangioblastoma. Patient underwent abdominal ultrasound to screen for lesions associated with Von Hippel-Lindau disease; this was normal.

6 months later, presents with tachycardia and flushing. Found to be hypertensive and had high urine catecholamines. MRI requested to look for pheochromocytoma.

MRI abdomen


There are bilateral adrenal masses - high signal on T2 weighted images and hyperenhancing following contrast administration. No renal, liver or pancreatic lesions.

Iodine 123 MIBG scan

Nuclear medicine

Tracer activity is noted bilaterally in the upper abdomen, more so on the left. Appearances in keeping with bilateral pheochromocytomas.

Case Discussion

vHL is a rare genetic disorder characterized by the development of both benign and malignant tumors in various systems, most notably within the CNS and in the abdomen.

The main features can be recalled using the mnemonic HIPPEL (taken from the article on vHL):

  • Haemangioblastoma
  • Increased risk of RCC
  • Phaeochromocytoma
  • Pancreatic lesions (cysts, cystadenomas, cystadenocarcinomas)
  • Eye dysfunction (retinal hemangioblastomas)
  • Liver, renal and pancreatic cysts

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